Pathology Flashcards
(143 cards)
0
Q
Describe Viral Meningitis (Symptoms, Cells, Glucose, and Protein)
A
Symptoms: Less acute than bacterial, less severe symptoms
Cells: Lymphocyte predominant
Glucose: Decreased
Protein: Slightly elevated
1
Q
Describe Bacterial Meningitis (Symptoms, Cells, Glucose, and Protein)
A
Symptoms: High fever, HA, nuchal rigidity, Kernig and Brudzinski signs
Cells: PMN predominant
Glucose: Decreased
Protein: Increased
2
Q
Describe Fungal Meningitis (Symptoms, Cells, Glucose, and Protein)
A
Symptoms: Seen in Immunocompromised patients
Cells: Lymphocyte predominant
Glucose: Normal
Protein: slightly elevated
3
Q
What is Kernig’s Sign?
A
Pain elicited while straightening knee with hip flexed at 90degrees
4
Q
What is Brudzinski’s Sign?
A
Patient flexes knees in response to passive flexion of neck.
5
Q
List layers that needle of lumbar puncture goes through
A
Skin–> subcutaneous tissue–> supraspinaous/interspinous ligaments–> Ligamentum flavum–> Epidural fat–> Epidural space–> Dura mater–> Arachnoid space–> Subarachnoid space!
6
Q
What is Noncommunicating Hydrocephalus?
A
Ventricles do NOT communicate with subarachnoid space, but CSF production continues
7
Q
What is Communicating Hydrocephalus?
A
Ventricles DO communicate with subarachnoid space. Can arise from:
1) CSF overproduction
2) CSF obstruction
3) poor CSF absorption
8
Q
What is Normal Pressure Hydrocephalus? (Symptoms)
A
Chronic form, with equilibrium between production/absorption. Often proceeded by high-pressure phase.
Symptoms: “Wet, wacky, and wobbly”–> Urinary incontinence, progressive dementia, and ataxic gait.
9
Q
What is Hydrocephalus ex vacuo?
A
expansion of ventricular volume secondary to loss of brain tissue
10
Q
What is Pseudotumor Cerebri? (Symptoms, causes)
A
Benign intracranial HTN. Increased resistance to CSF outflow. Seen in obese women.
Symptoms: HA, visual changes. Slit-like ventricles on imaging
11
Q
Where do eyes look in Stroke/ Seizure?
A
Stroke: TOWARD stroke
Seizure: AWAY from a seizure
12
Q
What is the difference between Dyslexia and Alexia?
A
Dyslexia: congenital
Alexia: acquired
13
Q
What is Apraxia?
A
inability to carry out learned movements (combing hair, brush teeth)
14
Q
What kind of memory loss results from Hippocampal lesion?
A
Anterograde memory loss
15
Q
What can lesion to Amygdala cause? What is this syndrome called?
A
Kluver-Bucy sundrome.
Psychic blindness, personality changes (abnormal docility), hyperorality, hypersexuality
16
Q
What is difference between lesion proximal and distal to CN VIII decussation?
A
Proximal to decussation and Sup. Olive–> Unilateral hearing loss
Distal to decussation and medullary cochlear nuc–> Bilateral hearing diminished, WITHOUT hearing loss
17
Q
What is the clinical presentation of hydrocephalus? (3)
A
1) Headache
2) vomiting without nausea
3) papilledema
18
Q
How do you treat hydrocephalus?
A
ventricular-peritoneal shunt
19
Q
What can cause Dandy-Walker Malformation?
A
Riboflavin inhibitors, posterior head trauma, viral infection (rubella, CMV)
20
Q
Which form of hydrocephalus is common in babies?
A
Congenital aqueduct of Sylvius Stenosis (between 3rd and 4th ventricles)
21
Q
Hydrocephalus Ex Vacuo in Elderly (Cause, symptoms, Diag, Tx)
A
Cause: significant neuronal loss
Symptoms: HA, vomiting w/o nausea, papilledema
Diagnosis: MRI, CT
Tx: None
22
Q
Meningitis
A
Cause: Inflamm of leptomeninges
Symptoms: meningismus, nuchal rigiditty, HA, fever, vomiting w/o nausea, papilledema
Diagnosis: CT prior to Lumbar puncture to prevent tonsillar herniation
Tx: Abx
23
Q
What MUST you do before performing lumbar puncture in patient that is thought to have increased intracranial pressure (ICP)?
A
inspection for papilledema and loss of venous pulsations on PE and CT–> to prevent tonsillar herniation wit decreased ICP
24
Who are at higher risk for meningitis? How can meningitis cause hydrocephalus?
elderly, very young (<3yo) and those living in close quarters. Scarring of meninges and edema can cause hydrocephalus.
25
Subarachnoid hemorrhage (cause, symptoms, diagn, tx, prognosis)
Cause: ruptured aneurysm (Ant. comm artery) or congenital malformation
Symptoms: "Worst HA of my life", CN III palsy, decreased levels of consciousness, hydrocephalus symptoms, bloody/xanthochromic spinal tap
Diag: CT, xanthochromic CSF
Tx: surgical excision of aneurysm, or fill with metal coil to PREVENT
Prognosis: 45% die w/in 1month.
26
Normal Pressure Hydrocephalus (Cause, symptom, diagnosis, treatment)
Cause: meningitis, subarachnoid hemorrhage, atherosclerosis--> reduced resorption of CSF.
Symptoms: bladder incontinence, dementia, and ataxia ("Magnetic gait"). NO papilledema or HA because ICP not increased.
Diag: CT/MRI (ventriculomegaly w/o proportional sulcal atrophy or increased CSF), clinical signs
Tx: shunt
27
For cardiovascular disorders, when do you use CT and when do you use MRI?
CT: hemorrage/ blood
MRI: infarction
28
What happens after thrombotic stroke (Pale Infarction)? (days, weeks,...)
Liquifactive necrosis due to lack of reperfusion
1-2 Days: edema, loss of demarcation between white/gray matter, myelin breakdown
Weeks: cystic area and gliosis
29
What is the Treatment for a pale infarction? (Short and long-term, side effects)
Short-term: IV Tissue Plasminogen Activator within 3HRS after onset of symptoms--> dissolve clot and enable reperfusion
IV tPA NOT given if CT shows existing hemorrhage, or is time of onset is unclear
SIDE EFFECT: hemorrhage
Long-term: aspirin, statin, rehabilitation
30
What is thought to be the cause of Embolic stroke (hemorrhagic infarction)?
Lysis of embolic material--> blood extravasation through damaged blood vessel (Reperfusion injury)
31
How does Embolic stroke present? Treatment?
Symptoms: clinically indistinguishable from thrombotic stroke
Tx: underlying conditions causing emboli, anticoagulants, rehabilitation.
32
Which symptom can help distinguish between hemorrhage and infarction?
Vomiting is much more common with hemorrhage than infarction.
33
What is Binswanger Disease?
Widespread degeneration of cerebral white matter, secondary to vascular lesion (HTN, atherosclerosis, multiple strokes)
Symptoms: gait disorder, dementia, pseudobulbar state
Variant of multi-focal dementia.
34
Epidural Hemorrhage (Etiology, Presentation, Tx, prognosis)
Etiology: Middle Meningeal Artery rupture
Presentation: "Talk and die". Loss of consciousness with lucid interval, bradycardia with increased sys BP, on CT blood does NOT cross suture lines, dilated pupil
Tx: hematoma clot evacuation and cauterize MMA
Prognosis: brain damage, death can occur with increased ICP, uncal herniation, resp. arrest
35
Subdural Hemorrhage (Etiology, Presentation, Tx, prognosis)
Etiology: Bridging veins rupture, brain atrophy, abrupt deceleration
Presentation: delayed onset due to low-press venous sys, fluctuating level of consciousness, on CT blood CROSSES suture lines
Tx: removal of hematoma by craniotomy
Prognosis: w/o treatment, cerebral compression, temporal lobe-tentorial herniation--> death
36
Subarachnoid Hemorrhage (Etiology, Presentation, Tx, prognosis)
Etiology: rupture of aneurysm, arteriovenous malformation
Presentation: "worst HA of my life" occipital, bloody/xanthocromic spinal tap, impaired CSF resorption--> hydrocephalus
Tx: surgical excision of aneurysm or fill with metal (prevention)
37
Parenchymal (Etiology, Presentation, Tx, prognosis)
Etiology: diabetes mellitus, tumor, HTN, amyloid angiography, Charcot-Bouchard macroaneurysm
Presentation: basal ganglia, thalamus, internal capsule most affected--> hemiplegia, contralateral sensory loss, vomiting, inability to sit/walk/stand (damage to cerebellum)
Tx: ventilation, monitor ICP and BP (keep below 110mmHg), surgical evacuation of cerebellar hematomas
Prognosis: depends on location. 30-35% die w/in month.
38
What are some causes of lacuna infarcts? Tx and prognosis?
Hyaline arteriosclerosis secondary to HTN and diabetes mellitus
Tx: Lower BP, control DM, aspirin
Prognosis: generally fair to good.
39
How will a MCA stroke present?
Parietal Lobe: contralateral hemianesthesia (face/arm worse than leg)
Frontal Lobe: contralateral hemiplegia (face/arm worse than leg)
Temporal Lobe: homonymous quadrantanpia
If Dominant Hemisphere (usually LEFT): aphasia
If Nondominant Hemisphere (usually RIGHT): sensory neglect and apraxia
40
How will an ACA stroke present?
Parietal: contralateral hemianethesia (leg worse than face/arm)
Frontal: contralateral hemiplegia (leg worse than face/arm)
Medial frontal: urinary continence, grasp refelx
41
How will a PCA stroke present?
Occipital homonymous hemianopia w/ macular sparing
42
How will an AICA stroke present?
"Lateral pontine syndrome"
Ipsilateral: paralysis of face movt (CN VII), paralysis of conjugate gaze to side of lesion (CN VI), face pain/temp, Horner syndrome, hearing loss/vertigo/nausea, vomiting, nystagmus away from lesion, dystaxia
Contralateral: loss of pain/temp in body
43
How will a PICA stroke present?
"Wallenberg Syndrome/ Lateral Medullary Syndrome"
Ipsilateral: limb ataxia, intention tremor (Inf. Cerebellar peduncle), vertigo/nausea/vomiting/nystagmus away from lesion (CN VIII nuc.), paralysis of larynx/pharynx/palate (Nuc. Ambiguus), facial temp/pain loss (CN V), Horner (descending hypothalamic)
Contralateral: loss of pain/temp in body
44
What if right-handed patient presents with aphasia- which side is lesion?
LEFT hemisphere b/c usually dominant hemisphere. This is true for most left-handed patients also, but not all (some have language area on right hemisphere)--> have to confirm with CT/MRI.
45
How to treat Hemiballismus? Prognosis?
Tx: Haloperidol
Prognosis: weeks w/o tx can lead to exhaustion and death
46
What is Broca aphasia? Location? Presentation?
Location: Inferior frontal gyrus.
Presentation: motor/ nonfluent/expressive aphasia with good comprehension. Patients are aware--> frustrating. Often accompanied by contralateral hemiparesis of right lower face/arm
47
What is Wernicke aphasia? Location? Presentation? Prognosis?
Location: Sup. temporal gyrus.
Presentation: Sensory/fluent/auditory aphasia w/ impaired comprehension, neologisms, paraphasic errors. Unaware of defect. Often with contralateral visual defects (Meyer's Loop), alexia
Prognosis: less likely to return to normal than Broca
48
What would lesion to Arcuate Fasciculus look like?
```
Conduction aphasia (Arcuate fasciculus connects Broca and Wernicke)
Presentation: Poor repetition, intact comprehension, fluent speech, cannot name objects
```
49
What is Global aphasia?
Presentation: speech and comprehension affected. Often accompanied by right hemiplegia, hemianesthesia, homonymous hemianopia
50
Amyotrophic Lateral Sclerosis (Pathology, Presentation, and Tx/Prognosis)
Pathology: Neurodegeneration of UMN and LMN
Presentation: LMN and UMN signs with sensory sparing
Tx: Riluzole delays onset of ventilator-dependence (3-5mts). Rapidly fatal.
51
Werdnig-Hoffman Disease. (Pathology, Presentation, and Tx/Prognosis)
Pathology: inherited LMN disease. Degeneration of Ant. Horn. NO UMN involvement.
Presentation: "Floppy baby syndrome", tongue fasciculations, LMN signs
Tx/Prognosis: Average age is 7yo at death.
52
Poliomyelitis. (Pathology, Presentation, and Tx/Prognosis)
Pathology: Degeneration of Ant. Horn
Presentation: Follows infection with Polio--> first infects oropharynx and intestines
Tx: strict bed rest to delay paralysis, ventilation
53
Alzheimer's Disease. (Pathology, Presentation, and Tx/Prognosis)
Pathology: B-amyloid plaques in neurons and cerebral blood vessels. Mutations in Presenilin 1/2, APOE4, p-APP. Decrease in ACh.
Presentation: mental deterioration, short-term memory loss, anosmia, decline in executive fcn, SPARES sensory/motor fcn.
Tx: AChE inhibitors (increase ACh), NMDA receptor antagonists (decrease glutamate-toxicity)
Prognosis: 50% expected due to cardio/resp problems and starvation
54
Pick Disease. (Pathology, Presentation, and Tx/Prognosis)
Pathology: pick bodies (tau protein), straight filaments, loss of white matter
Presentation: personality changes (first sign), dementia, aphasia, memory loss
Tx: (similar to Alzheimer's) AChE inhibitors and NMDA receptor antagonists
55
What is Multi-infarct (vascular dementia)? (Pathology, Presentation, and Tx/Prognosis)
2nd most common cause of dementia
Pathology: secondary to atherosclerosis
Tx: vascular prophylaxis and stroke prevention
56
What is Wilson Disease? (Pathology, Presentation, and Tx/Prognosis)
Pathology: Excessive Cu accumulation in liver, lenticular nucleus of basal ganglia, eyes. Decrease in Ceruplasmin and increase in urinary Cu.
Presentation: Onset at 20-30yr. Preceded by liver cirrhosis. Asterixis, dystonia, tremor, dementia, green-brown pigmentation of iris (Kayser-Fleischer rings)
Tx: Zinc, penicillamine (Cu chelators) and pyridoxine (prevent anemia), low Cu diet. Liver transplant curative.
57
What is Dementia Lewy bodies? (Pathology, Presentation, and Tx/Prognosis)
Pathology: widespread formation of Lewy bodies (a-synuclein aggregates)
Presentation: Parkinsonian WITH episodic delerium, visuospatial impairment, varying attention, undulating clinical course
Tx: Selegiline, MAO inhibitors
Prognosis: death due to immobility, poor nutrition, swallowing difficulty
58
What is Progressive Supranuclear Palsy?
Pathology: widespread neuronal loss and subcortical gliosis. SPARES cerebral and cerebellar cortices.
Presentation: difficulty with vertical eye gaze, pseudobulbar palsy, axial dystonia, bradykinesia w/o tremor. Memory and intellect INTACT.
Tx: No definitive tx.
Prognosis: death 6-10yr
59
Olivopontocerebellar atrophy (Pathology, Presentation, and Tx/Prognosis)
Pathology: loss of Purkinje cells, flattening of pons and enlarged 4th ventricle
Presentation: ataxia, dysarthria, intention tremor, generalized rigidity, dementia
Tx: progressive degeneration--> falling and aspiration pneumonia
60
Friedrich ataxia. (Pathology, Presentation, and Tx/Prognosis)
"Hereditary Ataxia"
Pathology: Autosomal recessive (GAA repeat, Frataxin gene).
Presentation: Onset w/in first 10yrs of life. atrophy of spinal cord, damage to dorsal columns, spinocerebellar tracts, lateral corticospinal tracts. Loss of proprioception, deep tendon reflexes, + Babinski
Prognosis: poor.
61
Progressive Multifocal Leukoencephalopathy (PML)
Pathology: VIRAL. Reactivation of latent JC (John Cunningham) virus in Oligodendrocytes. Especially in AIDS patients (immunosuppression).
Presentation: hemiparesis, aphasia ,cortical blindness, conjugate gaze abnormalities. RAPID progression.
Prognosis: severe dementia, death w/in 6mts-1yr
62
Multiple Sclerosis
Pathology: inflamm autoimm to Oligodendrocytes
Presentation: scanning speech, intention tremor, internuclear ophthalmoplegia, nystagmus. Often relapsing, remitting.
Diagnosis: Labs (increased OLIGOCLONAL IgG BANDS, myelin basic protein, leukocytes) in CSF, plaques on MRI
Treatment: High-dose steroids (Acute) Interferon-Beta (Long-term), Natalizumab.
63
Acute Disseminated (Postinfectious) Encephalomyelitis
Pathology: *follows infection* with measles, mumps, rubella, chickenpox.
Presentation: Onset much sooner than SSPE. Hx of recent infection, irritable, lethargic, mental status changes, seizures
Tx: high dose corticosteroids, Plasmapharesis, IVIg
64
What is the difference between Subacute Sclerosing Panencephalitis and Acute Disseminated (Postinfectious) Encephalitis?
timing of symptoms. ADPE: much sooner
65
Guillain-Barre Syndrome
Pathology: inflamm autoimm against PNS myelin. Assoc with infections (HSV, Camppylobacter jejuni, Mycoplasma pneu, flu vaccine)
Presentation: NO fever. Symmetrical ASCENDING muscle weakness, parasthesia
Tx: *resp support is CRITICAL*, plasmapharesis, IVIg
66
Central Pontine Myelinolysis (CPM)
"Locked-In syndrome"!!!
Pathology: rapid correction of hyponatremia (re-feeding syndrome, alcoholics, liver disease)--> shrinking of neurons--> demyelination
Presentation: rapid-onset of quadriparesis, dysarthria, dysphagia
Prognosis: death is common
67
What is the difference between CPM and MS?
CPM does NOT have inflammation.
68
What is Status Epilepticus? Tx?
seizures lasting more than 20min w/o regaining consciousness
tonic-clonic
sudden loss of consciousness
rhythmic contraction of limbs
Tx: benzodiazapines (IMMEDIATE), phenytoin (LONG-TERM)
69
What can cause cerebral edema? When does it occur?
Cytotoxic: secondary to hypoxia and hyponatremia
Vascular: increased permeability of vessels from inflamm.
Occurs 2-4days after infarction
70
What is Cushing Reflex? When does it occur?
bradycardia (parasympathetic)+ HTN (sympathetic)
| occurs with ICP
71
How can EEG help diagnose seizure?
spike and wave complex--> signifies hypersynchronocity
72
What is Partial seizure? (simple vs. complex)
Simple: consciousness intact, motor/sensory/autonomic/psychic components
Complex: alteration of consciousness
73
What are the different types of Generalized seizures?
Absence (petit mal, no postictal confusion)
Myoclonic
Tonic-clonic (has postictal phase- lethargy and confusion)
Tonic
Atonic ("Dropping", mistaken for fainting)
74
May-White Syndrome
Familial progressive myoclonic epilepsy
| w/ lipoma, ataxia, deafness
75
What are the signs of ICP (increased cranial pressure)?
papilledema, vomiting, sinus bradycardia + HTN (Cushing reflex), decreased level of consciousness
76
How do you treat patient with head trauma? Why?
Intentional hyperventilation--> RESPIRATORY ALKALOSIS--> cerebral vessel constriction --> decreases cerebral blood volume and permeability
To decrease risk of cerebral edema
77
What does Respiratory Acidosis induce?
vasodilation and increased vessel permeability--> enhances cerebral edema
78
What is the difference between Coup and Contrecoup? Which is worse?
Coup: site of impact injury
Contrecoup: opposite side of impact (more devastating because requires more impact)--> axonal damage
79
Astrocytomas in adults vs. children
Account for 70% neuroglial tumors
Adults: frontal
Children: cerebellum
80
DDx for bitemporal hemianopsia from sella turcica in adults/children?
Adults: Pituitary adenoma
Children: craniopharyngioma
81
What is the most common form of pituitary adenoma?
Prolactin-secreting adenoma
82
Primary Brain tumors (adult vs. childhood)
Seldom undergo metastasis
Adult: Supratentorial
Childhood: Infratentorial
83
Where do adult brain tumors metastasize from? (in order)
lung, breast, skin (melanoma), kidney, GI, thyroid
84
What is the most common primary brain tumor in adults? Prognosis?
Glioblastoma. <1 yr life expectancy
85
Glioblastoma
"Pseudopalisading" tumor cell border
central areas of necrosis and hemorrhage
Most common primary brain tumor in adults
Astrocytoma--> visualize with GFAP
86
Meningioma
*most common benign primary CNS tumor in adults
Pathology: from arachnoid cells, Psammoma bodies
Presentation: convexities of hemispheres, spastic paresis and urinary incontinence
Tx: resectable
87
What is the most common benign primary CNS tumor in adults?
Meningioma
88
Shwanomma-neurofibroma
3rd more common benign primary tumor
Pathology: commonly occurs in neurofibromatosis II (NF 2). Cerebellopontine angle--> usually present w/ deafness
Tx: Surgical resection
89
Oligodendroglioma
Slow-growing, most often in frontal lobe
"Fried egg cells"
Tx: surgical resection
90
Pinealoma
"Parinaud syndrome"
| Tx: resection
91
Pilocytic astrocytoma. Prognosis?
Pathology: benign glioma. "Rosenthal fibers" (eosinophilic corkscrew fibers)
Presentation: most often in cerebellum (posterior fossa)
Prognosis: Good
92
Ependyoma
Pathology: Commonly in 4th ventricle
Presentation: can cause hydrocephalus--> nausea, vomiting, nuchal rigidity, increased ICP
93
Medulluloblastoma
Pathology: highly malignant tumor.
Presentation: Cerebellar vermis. Can compress 4th ventricle--> hydrocephalus and increasing ICP.
Tx: radiosensitive. Can progress to cerebellar herniation w/o surgery
94
Retinoblastoma
Malignant retinal tumor
sporadic--> Unilateral
bilateral--> Rb gene deletion
Tx: radiotherapy, cryotherapy, chemo, enucleation
95
Hemangioblastoma
*assoc. with von Hippel-Lindau Syndrome
foamy cells and high vascularity
Tx: resection
96
Craniopharyngioma
*Most common childhood SUPRETENTORIAL tumor*
Benign
Derived from Rathke Pouch (ectoderm resembling tooth enamel)
Presentation: growth failure, papilledema, compression of pituitary and/or optic chiasm
Tx: resection
97
Psammoma bodies (What is it? Assoc. with which cancers?)
Laminated calcifications
Associated with: Papillary adenocarcinoma (thyroid), Papillary Serous Cystadenocarcinoma (ovary), Meningioma, Mesothelioma
98
How do you visualize astrocytes/ astrocytomas?
Stain for Glial Fibrillary Acidic Protein (GFAP) , an intermediate filament
99
What are Meningiomas assoc with? What else is this disease assoc with?
Neurofibromatosis (NF)
NF 2 also assoc. with bilateral schwannomas
100
What is Neurofibromatosis assoc with? (2)
Bilateral Schwannoma and Meningiomas
101
Compare tx for hypersecreting prolactin, GH, ACTH, and TSH tumors
Prolactin: DA-agonists or surgical resection
| All other: surgical resection
102
Brown-Sequard Syndrome (also, Tx and prognosis)
Hemisection of sp. cord
Motor: IPSILATERAL UMN below lesion, LMN lesion at level of lesion
Sensation: IPSILATERAL loss of fine touch/vibration/ proprioception at and below lesion, CONTRALATERAL loss of pain/temp below lesion, loss of ALL sensation at level of lesion
Tx: high dose steroids
Prognosis: poor
103
Upper Extremity Nerve Injury (radial, median, ulnar, axillary, musculocutaneous)
Radial (C5-C8)--> shaft of humerus
Median (C5-T1)--> Supracondylar region of humerus, Carpel tunnel syndrome, slashing of wrist
Ulnar (C8-T1)--> Medial epicondyle
Axillary (C5 and C6)--> surgical neck of humerus, anterior shoulder dislocation. On physical exam (palpable depression under acromion)
Musculocutaneous (C5 and C6)--> decreased supination, loss of function of biceps, brachialis, coracobrachialis
104
Anterior Compartment Syndrome
Pathology: increased pressure--> compression of Deep Peroneal Nerve and vasculature--> Foot Drop
105
CNS lymphomas usually result from metastasis of what?
High-grade Hodgkin lymphoma with B-cell origin
106
What is Primary CNS lymphoma associated with?
AIDS.
EBV lymphoma is most common cerebral tumor in AIDS pts.
MRI--> single ring-enhancing lesion. Toxoplasmosis (usually multiple lesion) must be ruled out.
107
What is the difference between Craniopharyngioma and Pituitary adenomas?
Craniopharyngiomas: symptoms from interruption of infundibulum
Pituitary Adenomas: Symptoms from combination of aberrant tumor cell hormone secretion AND mass effect
108
What is subependymal astrocytoma pathognomonic for?
Tuberous Sclerosis
| Also, cardiac myomas and renal angiomyolipomas
109
Which familial syndromes are associated with Pituitary adenomas?
Multiple Endocrine Neoplasia (MEN 1)
McCune Albright syndrome
Familial Acromegaly
110
Poliomyelitis
Pathology: fecal-oral transmission of Poliovirus. First replicates in oropharynx/small int. Destruction of anterior horn--> LMN destruction
Presentation: (Acute) Malaise, HA, fever, nausea, abdominal pain, sore throat. (Later) LMN signs.
Diag: CSF--> lymphocytic pleocytosis, slight elevation of protein.
Tx: Bed rest to prevent paralysis, ventilator
Prognosis: low if bulbar involvement
111
Poliovirus Vaccines
Salk: inactive, parenteral
Sabine: active, oral. Small risk of disease, but confers mucosal immunity, which Salk does not.
112
Tabes Dorsalis (Tertiary Syphilis)
Pathology: degeneration of dorsal roots and columns (mainly lumbosacral, fasciculus gracilis). 15-20yrs after Treponema pallidum (Syphilis) infection
Presentation: Purely sensory- muscle power preserved. Ataxia, bilaterally impaired proprioception, Argyll Robertson pupils. Paresthesia (shooting pain).
Diagnosis: CSF--> pleocytosis, increased protein. Must follow venereal tests w/ specific syphilis tests.
Tx: penicilin
Prognosis: if untreated, paralysis, blindness, dementia
113
Which tests should you do to confirm Syphilis? Why?
Should follow Venereal Disease Research Lab tests with specific treponemal tests b/c VDRL tests can be + w/ autoimm diseases, mononucleosis, or hepatitis.
114
Subacute Combined Degeneration (Vit B12 deficiency)
Pathology: failure to absorb B12--> methylmalonyl-CoA and propionyl-CoA--> displaces succinyl-CoA in FA synthesis--> odd-chain FA into lipids/myelin--> demyelination of dorsal columns and corticospinal tract
Presentation: dementia, ataxic gait, spastic paresis, impaired position/vibration sense
Diagnosis: Serum cobalamin, MRI, Schilling Test
Tx: Vit. B12 supplements
115
What is the Schilling Test?
1) oral radiolabeled B12 + IM unlabeled B12--> measure urine B12 (should be high)
2) If first test abnormal--> rB12+ intrinsic factor--> measure urine B12
3) If still no B12 in urine--> rB12+ Abx (to rule out bacterial overgrowth)--> measure urine B12
4) If still no B12 in urine--> rB12 + pancreatic enz (rule our pancreatitis)--> measure urine B12
116
What can cause Pernicious Anemia? What can PA cause?
autoimmune disorder
Ab against Intrinsic factor and/or gastric parietal cells
--> Subacute Combined Degeneration and Megaloblastic anemia
117
Syringomyelia
Pathology: enlargement of central canal in sp. cord--> damage to Ant. Commissure--> spinothalamic tract damaged
Presentation: loss of pain/temp sensation in UE (C8-T1). "Cape and Shawl" distribution. May also damage Ant. Horn--> LMN defects.
Diagnosis: MRI
Tx: If have Arnold-Chiari--> shunt
Prognosis: Poor.
118
What is a common presentation of Syringomyelia?
Repeatedly burning their hands. "Cape and shawl" distribution of loss of pain/temp sensation.
119
Thoracic Outlet Syndrome (Klumpke Palsy)
Pathology: embryologic defect in cervical rib--> compression of subclavian artery and inf. trunk of brachial plexus (C8, T1). Also seen in weightlifters
Presentation: Atrophy: thenar and hypothenar eminence (median and ulnar n.), interosseous musc (ulnar n.), sensory deficits of forearm and hand (ulnar and medial cutaneous n.). Disappearance of radial pulse when head is rotated
Tx: Ant. scalenectomy (careful to avoid phrenic n.), remove cervical rib
Prognosis: symptoms may recur due to scarring/ fibrosis
120
How do you tell the difference between ALS and Syringomyelia?
ALS: NO sensory deficits
121
What can cause Toxin-assoc peripheral neuropathy?
alcohol abuse, exposure to heavy metals, diptheria
122
What does the Radial N. innervate
```
"BEST"
B= brachiocradialis
E= extensors
S= supinator
T= triceps
```
123
Describe the 2 types of peripheral neuropathy
1) Demyelination: segmental sensory loss/changes
2) Axonal degeneration: Axonal degeneration of sensory occurs FIRST (smaller fibers, more prone to damage)--> "Stocking and Glove" distribution b/c longer fibers more prone. Motor nerves--> fasciculations, atrophy.
124
Which nerves are more prone to axonal degeneration?
Sensory nerves (smaller fibers)
Long fibers
"Stocking-Glove" distribution
125
For meningitis, which pathogens are most common in NEONATES?
Group B Strep, E. coli, and Listeria monocytogenes
126
Charcot-Marie Tooth Disease
*Most common hereditary neuropathy*
Pathology: primarily affects peroneal nerve
Presentation: "inverted bottle" legs, foot drop, scoliosis, high/flat arch
Tx: physical therapy, surgical contractures for legs
Prognosis: no cure, progressive.
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For meningitis, which pathogens are most common in CHILDREN and TEENAGERS?
N. meningitidis
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For meningitis, which pathogens are most common in ADULTS and ELDERLY?
Step pneumoniae
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For meningitis, which pathogens are most common in NONVACCINATED INFANTS
H. influenza
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Most common viral source for meningitis? Who?
Coxsackievirus (Children)
| fecal-oral transmission
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Who gets fungal meningitis?
immunocompromised individuals
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What is the classic triad of meningitis? What else?
HA, nuchal rigidity, fever
| Also, photophobia, vomiting, altered mental status
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Which layer DON'T you go through for an Lumbar puncture? Which space do you go into?
pia. Go into subarachnoid space.
134
Complications from meningitis?
Death--> due to herniation from cerebral edema
| Hydrocephalus, hearing, loss, seizures,--> due to scarring/fibrosis
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Herniations (4)
Cingulate/ Subfalcine--> compresses Ant. Cerebral Artery
Uncal--> compressed crus cerebri, CN III, Post. Cerebral Artery--> infarction of occipital lobe. Rupture of Paramedian Artery--> Duret brain hemorrhage
Cerebellar tonsillar--> compression of medulla--> cardio-resp arrest
Transtentorial--> compression of brain stem--> coma, death
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Leukodrystrophy (Metachromatic)
Deficiency of arylsulfatase--> sulfatides (myelin) cannot be degraded--> accumulates in lysosomes (lysosomal storage disease)
Most common leukodystrophy
Autosomal recessive
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Leukodrystrophy (Krabbe)
Deficiency of galactocerebrosidase--> galactocerebroside and psychosine
Accumulates in macrophages
Autosomal recessive
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Multiple Sclerosis is more common in which people?
women, regions away from equator (due to environmental triggers that activate autoimmunity)
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Leukodrystrophy (Adrenoleukodystrophy)
Impaired addition of coenzyme A to long-chain FA--> Accumulation of FA damages Adrenal Glands AND white matter
X-linked
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What are the 2 timing features of Multiple Sclerosis?
relapsing and remission
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Subacute Sclerosing Panencephalitis
SLOWLY progressive, debilitating encephalitis
persistent infection of brain with MEASLES VIRUS
infection occurs in infancy--> signs in childhood
viral inclusion in gray AND white matter
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What can cause Progressive Multifocal Leukoencephalopathy?
Reactivation of JC virus
| May be linked to use of monoclonal Ab Tysabri in MS.
